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Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture
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ARTICLE IN PRESS IHJ Cardiovascular Case Reports (CVCR) xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
IHJ Cardiovascular Case Reports (CVCR) journal homepage: www.elsevier.com/locate/ihjccr
Case Report
Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture J.S.N. Murthy a,∗ , Rajani Gorantla b , T. Periyasamy c , Arunan Murali b , Mahesh Vakamudi d , B. Ranjith Karthekeyan d , Rajendiran Swaminathan e , Leena Dennis Joseph e a
Department of Cardiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India Department of Radiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India c Department of Cardio Thoracic & Vascular Surgery, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India d Department of Anesthesiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India e Department of Pathology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India b
a r t i c l e
i n f o
Article history: Available online xxx Keywords: Cardiac angiosarcoma Cardiac pseudoaneurysm Cardiac tumors
a b s t r a c t A 35-year-old female, who is a known case of right atrial angiosarcoma with giant pseudoaneurysm had been referred to us. CT angiogram was done and it showed signs of impending rupture. The diagnosis was confirmed by surgery and histopathology. Very few cases of cardiac rupture were reported in literature. © 2016 Cardiological Society of India. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Cardiac angiosarcoma is a rare aggressive primary malignant tumor of the heart and often difficult to diagnose clinically due to nonspecific signs and symptoms. Echocardiography is the first line of imaging; however, the advent of cross-sectional imaging with CT & MRI allows earlier detection of tumor and its complications along with distant metastasis. 2. Case report A 35-year-old female was evaluated for dyspnea and chest pain, 5 months back with chest X-ray, contrast-enhanced CT (CECT) thorax, and cardiac MRI. CT thorax showed thin-walled partially thrombosed right atrial aneurysm (Fig. 1a) with multiple nodules in the lung. Cardiac MRI also showed a thin-walled aneurysm sac from the right atrium (Fig. 1b). There was no soft tissue infiltration of the right atrium and no pericardial effusion. The diagnosis of giant right atrial aneurysm was made. Her workup for connective tissue disorders came negative. She was started on medication and advised for regular follow-up.
∗ Corresponding author. E-mail addresses: [email protected] (J.S.N. Murthy), [email protected] (R. Gorantla), [email protected] (T. Periyasamy), [email protected] (A. Murali), [email protected] (M. Vakamudi), [email protected] (B. Ranjith Karthekeyan), [email protected] (R. Swaminathan), [email protected] (L.D. Joseph).
She came to our institute with progressive worsening of dyspnea and increased chest pain. In our hospital, routine blood investigations, ECG, chest X-ray and 2D echocardiogram were done. Blood investigations revealed anemia. ECG showed normal sinus rhythm. Chest X-ray showed huge cardiomegaly with mediastinal widening (Fig. 2). Echo correlation revealed large collection communicating with anterolateral margin of right atrium with to and from color flow. Minimal pericardial effusion was also seen. However there was no cardiac tamponade. No echogenic mass was noted with in the cardiac chambers. In view of continuous breathing difficulty and pericardial effusion, CECT chest was done, which showed a large aneurysmal sac near the right atrial appendage medially and extending up to the lateral chest wall (Fig. 3a and b). Luminal thrombus was seen on the lateral aspect of the aneurysm sac. Focal areas of wall irregularity and defects were observed on the lateral aspect of the sac, a diagnostic sign of aneurysmal rupture (Fig. 3a). There was also extraneous compression upon the superior vena cava with thrombosis at veno-atrial confluence (Fig. 3c). Large pericardial hematoma (Fig. 3d) was seen. The rest of cardiac chambers appear normal. In the lung window multiple hypodense nodules of various sizes (Fig. 4) are seen in both lung fields. On comparison with previous CT & MRI (Fig. 1a and b) done 5 months back, there was significant increase in size of aneurysmal sac with features of impending rupture and also increase in the size and number of lung nodules. The possibility of the underlying primary malignant cardiac tumor causing spontaneous right atrial rupture and pseudoaneurysm formation with lung metastasis
http://dx.doi.org/10.1016/j.ihjccr.2016.09.002 2468-600X/© 2016 Cardiological Society of India. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/ licenses/by-nc-nd/4.0/).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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ARTICLE IN PRESS J.S.N. Murthy et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2016) xxx–xxx
Fig. 1. Selective axial sections from CECT (a) and four-chamber view of cardiac MRI (b) showing defect in the right atrium (arrow heads) and partially thrombosed aneurysm (long arrows).
Fig. 2. Chest X-ray PA view demonstrating huge cardiomegaly and mediastinal widening.
was suspected. Based on CT findings of impending aneurysmal rupture she was taken up for surgical intervention with high-risk consent. Surgery was performed under cardiopulmonary bypass with femoro femoral cannulation. Right atrium was found very friable and fenestrated. Blood in the aneurysmal sac was drained and large thrombus was evacuated (Fig. 5a). Biopsy was taken from the lung nodule (Fig. 5b). Right atrium was reconstructed with bovine pericardium and hemostasis secured. Patient was weaned off from cardiopulmonary bypass. Decannulation was done, but the patient continued to have diffuse mediastinal ooze with deranged coagulation parameters. She received multiple blood transfusions and was shifted to intensive care unit with chest open and ventilator support. On the next day, she went into low cardiac output syndrome and could not be revived. Histopathological examination showed a tumor with endothelial cell pleomorphism and mitosis. The pleomorphic tumor cells were seen surrounding vascular lamina, suggesting a high-grade malignancy of vascular origin (Fig. 6a). This was confirmed by
Fig. 3. Axial sections of the CECT thorax showing extent of aneurysmal sac up to the lateral chest wall with partial thrombosis (a and b). Contour of sac demonstrating focal defects and irregularities (arrow heads in a and b). Distal SVC thrombosis at veno atrial confluence (arrow in c). Large pericardial hematoma was also seen (arrow in d).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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3
up to 50% of the benign masses.2 Among the malignant cardiac tumors, angiosarcoma is the common histological cell type. In contrast, secondary cardiac tumor or cardiac metastases are 20–40 times more common than primary masses3 and involve heart by hematogeneous or lymphatic spread and direct invasion from the mediastinum, vena cava, and right atrium.4 Cardiac angiosarcomas are common in the middle age men.2 Two-third of the lesions are located in the right atrium and pericardium followed by left atrium, right ventricle, and left ventricle.2 Right atrial angiosarcoma presenting with pseudoaneurysm formation is not reported in the literature frequently.2 Cardiac pseudoaneurysm can cause sudden cardiac death, but in our case, the patient was asymptomatic for 5 months. This could be due to a large thrombus in the aneurysm sac.5 However, pseudoaneurysm has to be intervened immediately due to high risk of rupture and cardiac tamponade as seen in our case. Fig. 4. Coronal reformatted CT thorax showing bilateral lung nodules.
3.2. Clinical presentation immunohistochemical positivity for Vimentin and CD34 (Fig. 6b). The diagnosis of high-grade angiosarcoma of the right atrium with lung metastasis was made. 3. Discussion 3.1. Epidemiology Primary cardiac tumors are rare lesions, with an incidence rate of less than 0.1% in an autopsy series.1 They can be benign or malignant. Myxoma is the most common tumor in adults accounting
The signs and symptoms are usually nonspecific and depend upon the location and extent of the tumor. They can present with chest pain, shortness of breath, malaise, fever, and right heart failure but most commonly present at advanced stage with metastasis and prognosis is invariably poor. If tumor fragments enter pulmonary circulation, it can cause symptoms consistent with pulmonary embolism.6 Cardiac rupture with pseudoaneurysm formation can also occur which is very rare. These patients can present with cardiac tamponade and can have sudden cardiac death.
Fig. 5. Intraoperative image showing the blood clot (a) and lung nodule (arrow in b).
Fig. 6. Histopathology sections from the resected specimen showing pleomorphic tumor cells surrounding vascular lumina (a). Tumor cells showing CD 34 positivity by immunohistochemistry (b).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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3.3. Imaging features Imaging features depend upon the morphological type of angiosarcoma. The first and common type is well-defined mass protruding in to the cardiac chamber, most commonly the right atrium. Second and less common type is diffuse infiltrating mass along the pericardium which can cause necrosis and cardiac rupture,7 as in our case. Role of imaging is to confirm the location of the primary tumor and evaluation of the metastasis. The common sites of metastasis are lungs, liver, lymph nodes, adrenal glands and spine.8 Metastasis is common in 66–89% of patients at the time of diagnosis.3 2D echocardiography is the first line of investigation tool for evaluating cardiac masses and anatomy in multiple planes. Most of them are seen as mural masses within the lumen or as infiltration mass of the pericardium or as discontinuity in the wall with pseudoaneurysm formation8 as in our case. However, it has limitations to demonstrate mediastinal and extracardiac involvement. Chest radiography may show cardiomegaly, pulmonary metastasis, hilar lymphadenopathy, and pleural effusion.7 Cross-sectional studies like cardiac MRI and CT have good temporal and spatial resolution respectively and demonstrate clearly the extent of the mass. They also show areas of hemorrhage and necrosis within the mass, infiltration of adjacent structures, and distant metastasis. In cardiac rupture, size of the defect, continuity of pseudoaneurysm, and thrombus in the atrial wall can be accurately described.7 The wall irregularity and focal discontinuity of the aneurysm cavity seen in our case on contrast CT, which are signs of impending rupture, has helped the surgeon to take decision. The suspicion of sarcoma should prompt early management as the survival rate is very less, ranging from 12 to 30 months with multimodality treatment and also have rapid progression.10 Treatment of angiosarcoma is mostly of multimodality approach (surgery, chemotherapy and radiotherapy) due to rapid growth, local invasion, and early metastasis at the time of presentation.9 In limited disease, radical resection followed by adjuvant radiotherapy and chemotherapy is the standard treatment. But in locally advanced tumor and metastasis prior treatment with
chemotherapy and radiotherapy may lead to shrinkage of the mass, allowing for radical surgery. Current gold standard chemotherapy agent is doxorubicin-based regimes.11 4. Conclusion Cardiac angiosarcomas are rare cardiac tumors with poor prognosis due to its advanced stage at the time of presentation. This article highlights the role of cross-sectional imaging for accurately diagnosing the cardiac and extracardiac extent of the tumor. Conflicts of interest The authors have none to declare. References 1. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993;117(10):1027–1031. 2. Sakaguchi M, Minato N, Katayama Y, Nakashima A. Cardiac angiosarcoma with right atrial perforation and cardiac tamponade: Case report. Ann Thorac Cardiovasc Surg. 2006;12(2):145–148. 3. Burke A. Tumors of the Heart and Great Vessels. Atlas of Tumor Pathology. vol. 16. Armed Forces Institute of Pathology; 1996:171–179. 4. Longo R, Mocini D, Santini M, et al. Unusual sites of metastatic malignancy: Case 1. Cardiac metastasis in hepatocellular carcinoma. J Clin Oncol. 2004;22(24):5012–5014. 5. Serra V, Moura L, Almeri C, et al. Roturada auricula direia. Rev Port Cardiol. 2004;5:731–736. 6. ElBardissi AW, Dearani JA, Daly RC, et al. Embolic potential of cardiac tumors and outcome after resection: a case–control study. Stroke. 2009;40(1):156–162. 7. Best AK, Dobson RL, Ahmad AR. Cardiac angiosarcoma: best cases from the AFIP. Radiographics. 2003;23:S141–S145. 8. Satou Y, Nakagawa Y, Miki H, Suzuki H, Takahashi M. Cardiac angiosarcoma with ruptured right atrium diagnosed by echocardiography. Chest. 1991;100(1):274–275. 9. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer. 1986;57:852–859. 10. Ostrowski S, Marcinkiewicz A, Ko´smider A, Jaszewski R. Sarcomas of the heart as a difficult interdisciplinary problem. Arch Med Sci. 2014;10(1):135–148. 11. Minichillo S, Pantaleo MA, Nannini M, Coccolo F, Biasco G, Brand G. Efficacy of weekly docetaxel in locally advanced cardiac angiosarcoma: case report. BMC Res Notes. 2015;8:325.
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
ARTICLE IN PRESS IHJ Cardiovascular Case Reports (CVCR) xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
IHJ Cardiovascular Case Reports (CVCR) journal homepage: www.elsevier.com/locate/ihjccr
Case Report
Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture J.S.N. Murthy a,∗ , Rajani Gorantla b , T. Periyasamy c , Arunan Murali b , Mahesh Vakamudi d , B. Ranjith Karthekeyan d , Rajendiran Swaminathan e , Leena Dennis Joseph e a
Department of Cardiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India Department of Radiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India c Department of Cardio Thoracic & Vascular Surgery, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India d Department of Anesthesiology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India e Department of Pathology, Sri Ramachandra University, Porur, Chennai 600116, Tamil Nadu, India b
a r t i c l e
i n f o
Article history: Available online xxx Keywords: Cardiac angiosarcoma Cardiac pseudoaneurysm Cardiac tumors
a b s t r a c t A 35-year-old female, who is a known case of right atrial angiosarcoma with giant pseudoaneurysm had been referred to us. CT angiogram was done and it showed signs of impending rupture. The diagnosis was confirmed by surgery and histopathology. Very few cases of cardiac rupture were reported in literature. © 2016 Cardiological Society of India. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Cardiac angiosarcoma is a rare aggressive primary malignant tumor of the heart and often difficult to diagnose clinically due to nonspecific signs and symptoms. Echocardiography is the first line of imaging; however, the advent of cross-sectional imaging with CT & MRI allows earlier detection of tumor and its complications along with distant metastasis. 2. Case report A 35-year-old female was evaluated for dyspnea and chest pain, 5 months back with chest X-ray, contrast-enhanced CT (CECT) thorax, and cardiac MRI. CT thorax showed thin-walled partially thrombosed right atrial aneurysm (Fig. 1a) with multiple nodules in the lung. Cardiac MRI also showed a thin-walled aneurysm sac from the right atrium (Fig. 1b). There was no soft tissue infiltration of the right atrium and no pericardial effusion. The diagnosis of giant right atrial aneurysm was made. Her workup for connective tissue disorders came negative. She was started on medication and advised for regular follow-up.
∗ Corresponding author. E-mail addresses: [email protected] (J.S.N. Murthy), [email protected] (R. Gorantla), [email protected] (T. Periyasamy), [email protected] (A. Murali), [email protected] (M. Vakamudi), [email protected] (B. Ranjith Karthekeyan), [email protected] (R. Swaminathan), [email protected] (L.D. Joseph).
She came to our institute with progressive worsening of dyspnea and increased chest pain. In our hospital, routine blood investigations, ECG, chest X-ray and 2D echocardiogram were done. Blood investigations revealed anemia. ECG showed normal sinus rhythm. Chest X-ray showed huge cardiomegaly with mediastinal widening (Fig. 2). Echo correlation revealed large collection communicating with anterolateral margin of right atrium with to and from color flow. Minimal pericardial effusion was also seen. However there was no cardiac tamponade. No echogenic mass was noted with in the cardiac chambers. In view of continuous breathing difficulty and pericardial effusion, CECT chest was done, which showed a large aneurysmal sac near the right atrial appendage medially and extending up to the lateral chest wall (Fig. 3a and b). Luminal thrombus was seen on the lateral aspect of the aneurysm sac. Focal areas of wall irregularity and defects were observed on the lateral aspect of the sac, a diagnostic sign of aneurysmal rupture (Fig. 3a). There was also extraneous compression upon the superior vena cava with thrombosis at veno-atrial confluence (Fig. 3c). Large pericardial hematoma (Fig. 3d) was seen. The rest of cardiac chambers appear normal. In the lung window multiple hypodense nodules of various sizes (Fig. 4) are seen in both lung fields. On comparison with previous CT & MRI (Fig. 1a and b) done 5 months back, there was significant increase in size of aneurysmal sac with features of impending rupture and also increase in the size and number of lung nodules. The possibility of the underlying primary malignant cardiac tumor causing spontaneous right atrial rupture and pseudoaneurysm formation with lung metastasis
http://dx.doi.org/10.1016/j.ihjccr.2016.09.002 2468-600X/© 2016 Cardiological Society of India. Published by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/ licenses/by-nc-nd/4.0/).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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ARTICLE IN PRESS J.S.N. Murthy et al. / IHJ Cardiovascular Case Reports (CVCR) xxx (2016) xxx–xxx
Fig. 1. Selective axial sections from CECT (a) and four-chamber view of cardiac MRI (b) showing defect in the right atrium (arrow heads) and partially thrombosed aneurysm (long arrows).
Fig. 2. Chest X-ray PA view demonstrating huge cardiomegaly and mediastinal widening.
was suspected. Based on CT findings of impending aneurysmal rupture she was taken up for surgical intervention with high-risk consent. Surgery was performed under cardiopulmonary bypass with femoro femoral cannulation. Right atrium was found very friable and fenestrated. Blood in the aneurysmal sac was drained and large thrombus was evacuated (Fig. 5a). Biopsy was taken from the lung nodule (Fig. 5b). Right atrium was reconstructed with bovine pericardium and hemostasis secured. Patient was weaned off from cardiopulmonary bypass. Decannulation was done, but the patient continued to have diffuse mediastinal ooze with deranged coagulation parameters. She received multiple blood transfusions and was shifted to intensive care unit with chest open and ventilator support. On the next day, she went into low cardiac output syndrome and could not be revived. Histopathological examination showed a tumor with endothelial cell pleomorphism and mitosis. The pleomorphic tumor cells were seen surrounding vascular lamina, suggesting a high-grade malignancy of vascular origin (Fig. 6a). This was confirmed by
Fig. 3. Axial sections of the CECT thorax showing extent of aneurysmal sac up to the lateral chest wall with partial thrombosis (a and b). Contour of sac demonstrating focal defects and irregularities (arrow heads in a and b). Distal SVC thrombosis at veno atrial confluence (arrow in c). Large pericardial hematoma was also seen (arrow in d).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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3
up to 50% of the benign masses.2 Among the malignant cardiac tumors, angiosarcoma is the common histological cell type. In contrast, secondary cardiac tumor or cardiac metastases are 20–40 times more common than primary masses3 and involve heart by hematogeneous or lymphatic spread and direct invasion from the mediastinum, vena cava, and right atrium.4 Cardiac angiosarcomas are common in the middle age men.2 Two-third of the lesions are located in the right atrium and pericardium followed by left atrium, right ventricle, and left ventricle.2 Right atrial angiosarcoma presenting with pseudoaneurysm formation is not reported in the literature frequently.2 Cardiac pseudoaneurysm can cause sudden cardiac death, but in our case, the patient was asymptomatic for 5 months. This could be due to a large thrombus in the aneurysm sac.5 However, pseudoaneurysm has to be intervened immediately due to high risk of rupture and cardiac tamponade as seen in our case. Fig. 4. Coronal reformatted CT thorax showing bilateral lung nodules.
3.2. Clinical presentation immunohistochemical positivity for Vimentin and CD34 (Fig. 6b). The diagnosis of high-grade angiosarcoma of the right atrium with lung metastasis was made. 3. Discussion 3.1. Epidemiology Primary cardiac tumors are rare lesions, with an incidence rate of less than 0.1% in an autopsy series.1 They can be benign or malignant. Myxoma is the most common tumor in adults accounting
The signs and symptoms are usually nonspecific and depend upon the location and extent of the tumor. They can present with chest pain, shortness of breath, malaise, fever, and right heart failure but most commonly present at advanced stage with metastasis and prognosis is invariably poor. If tumor fragments enter pulmonary circulation, it can cause symptoms consistent with pulmonary embolism.6 Cardiac rupture with pseudoaneurysm formation can also occur which is very rare. These patients can present with cardiac tamponade and can have sudden cardiac death.
Fig. 5. Intraoperative image showing the blood clot (a) and lung nodule (arrow in b).
Fig. 6. Histopathology sections from the resected specimen showing pleomorphic tumor cells surrounding vascular lumina (a). Tumor cells showing CD 34 positivity by immunohistochemistry (b).
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002
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4
3.3. Imaging features Imaging features depend upon the morphological type of angiosarcoma. The first and common type is well-defined mass protruding in to the cardiac chamber, most commonly the right atrium. Second and less common type is diffuse infiltrating mass along the pericardium which can cause necrosis and cardiac rupture,7 as in our case. Role of imaging is to confirm the location of the primary tumor and evaluation of the metastasis. The common sites of metastasis are lungs, liver, lymph nodes, adrenal glands and spine.8 Metastasis is common in 66–89% of patients at the time of diagnosis.3 2D echocardiography is the first line of investigation tool for evaluating cardiac masses and anatomy in multiple planes. Most of them are seen as mural masses within the lumen or as infiltration mass of the pericardium or as discontinuity in the wall with pseudoaneurysm formation8 as in our case. However, it has limitations to demonstrate mediastinal and extracardiac involvement. Chest radiography may show cardiomegaly, pulmonary metastasis, hilar lymphadenopathy, and pleural effusion.7 Cross-sectional studies like cardiac MRI and CT have good temporal and spatial resolution respectively and demonstrate clearly the extent of the mass. They also show areas of hemorrhage and necrosis within the mass, infiltration of adjacent structures, and distant metastasis. In cardiac rupture, size of the defect, continuity of pseudoaneurysm, and thrombus in the atrial wall can be accurately described.7 The wall irregularity and focal discontinuity of the aneurysm cavity seen in our case on contrast CT, which are signs of impending rupture, has helped the surgeon to take decision. The suspicion of sarcoma should prompt early management as the survival rate is very less, ranging from 12 to 30 months with multimodality treatment and also have rapid progression.10 Treatment of angiosarcoma is mostly of multimodality approach (surgery, chemotherapy and radiotherapy) due to rapid growth, local invasion, and early metastasis at the time of presentation.9 In limited disease, radical resection followed by adjuvant radiotherapy and chemotherapy is the standard treatment. But in locally advanced tumor and metastasis prior treatment with
chemotherapy and radiotherapy may lead to shrinkage of the mass, allowing for radical surgery. Current gold standard chemotherapy agent is doxorubicin-based regimes.11 4. Conclusion Cardiac angiosarcomas are rare cardiac tumors with poor prognosis due to its advanced stage at the time of presentation. This article highlights the role of cross-sectional imaging for accurately diagnosing the cardiac and extracardiac extent of the tumor. Conflicts of interest The authors have none to declare. References 1. Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med. 1993;117(10):1027–1031. 2. Sakaguchi M, Minato N, Katayama Y, Nakashima A. Cardiac angiosarcoma with right atrial perforation and cardiac tamponade: Case report. Ann Thorac Cardiovasc Surg. 2006;12(2):145–148. 3. Burke A. Tumors of the Heart and Great Vessels. Atlas of Tumor Pathology. vol. 16. Armed Forces Institute of Pathology; 1996:171–179. 4. Longo R, Mocini D, Santini M, et al. Unusual sites of metastatic malignancy: Case 1. Cardiac metastasis in hepatocellular carcinoma. J Clin Oncol. 2004;22(24):5012–5014. 5. Serra V, Moura L, Almeri C, et al. Roturada auricula direia. Rev Port Cardiol. 2004;5:731–736. 6. ElBardissi AW, Dearani JA, Daly RC, et al. Embolic potential of cardiac tumors and outcome after resection: a case–control study. Stroke. 2009;40(1):156–162. 7. Best AK, Dobson RL, Ahmad AR. Cardiac angiosarcoma: best cases from the AFIP. Radiographics. 2003;23:S141–S145. 8. Satou Y, Nakagawa Y, Miki H, Suzuki H, Takahashi M. Cardiac angiosarcoma with ruptured right atrium diagnosed by echocardiography. Chest. 1991;100(1):274–275. 9. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer. 1986;57:852–859. 10. Ostrowski S, Marcinkiewicz A, Ko´smider A, Jaszewski R. Sarcomas of the heart as a difficult interdisciplinary problem. Arch Med Sci. 2014;10(1):135–148. 11. Minichillo S, Pantaleo MA, Nannini M, Coccolo F, Biasco G, Brand G. Efficacy of weekly docetaxel in locally advanced cardiac angiosarcoma: case report. BMC Res Notes. 2015;8:325.
Please cite this article in press as: Murthy JSN, et al, Right atrial angiosarcoma presenting as giant pseudoaneurysm with impending rupture, IHJ Cardiovasc Case Rep. (2016), http://dx.doi.org/10.1016/j.ihjccr.2016.09.002